著明な脳血管病変を合併した肝脳疾患類瘢痕脳型を疑わせる1剖検例

An Autopsy Case of the Suspicious "Pseudoulegyric Type" of the Hepatoctirebral Disease with the Cerebrovascular Lesions

I．まえがき

　肝脳疾患類瘢痕脳型については，すでに5剖検例がえられ^1）〜3），その臨床，病理知見について総括記述され，今後さらに多くの症例の蓄積による知見の補足，本態の追求が期待されている。従来，この型の脳所見として，血管の器質性変化はみられぬものの，循環障害性病変がひとつの特徴をなす点が指摘されているが，本例は類瘢痕脳型を思わせる肝，脳両所見と同時に，脳のいちじるしい動脈変化と，明らかにそれに基く循環障害性の局所性壊死巣をあわせもち，肝脳疾患，血管病変のいずれをその本態とみるべきか問題のある例である。ここですぐさま、その結論をのべることは難かしいとしても，類瘢痕脳型の定型例と比較し，その異同を考察する必要がある。

In a patient, 37 year-old, male, right hemiparesis and aphasias were firstly noted. Subsequently he developed in him tetraplegia, contracture of joints, dementia, severa convulsions and episodic disturbed consciousness. He finally emaciated and expired. The total duration of illness was 3 years and 2 months.

The postmortem examinations revealed slight fib-. rosis of the atrophic liver, atrophy of the spleen and a small ulcer of the descending colon. In the brain, irregularly-shaped, ulegyric softenings of the cortices and diffuse subcortical demyelination with a slight to moderate organizations-process were widespread and symmetrical bilaterally in the tem-poral lobes, except for the Ammon's horn and the hippocampal gyrus; severest in the left calcarine region; moderately in the inferior frontal gyrus. Both meningeal and intracerebral arteries and ar-terioles corresponding to the deteriorated regions showed the arteriosclerotic changes, such as fibro-hyalinously-degenerated vessel walls and intimal proliferation. Astrocytic nuclei, on the other hand, developed a transitional feature to Alzheimer's type-II glia and contained carmine-positive granules which were also disseminated in the periadventitial spaces, parenchyma, and cytoplasm and nuclei of the nerve cells. The above-mentioned findings we-re ubiquitously distributed in the grey matters.

A question as to whether the present example could be belonged to the category of the "pseudo-ulegyric type" of the hepatocerebral disease or the cerebral arteriosclerosis has been discussed. Con-sequently it may be assumed that the fundamental process of the present case com.prized the organic cerebrovascular lesions in association with the cer-tain hepatogenic factors.